Congenital extrahepatic portosystemic shunt,also known as Abernethy malformation, is a rare congenital malformation. It causes shunting of blood through a communication between the portal and systemic veins such as a ...Congenital extrahepatic portosystemic shunt,also known as Abernethy malformation, is a rare congenital malformation. It causes shunting of blood through a communication between the portal and systemic veins such as a patent ductus venous. We report 3 cases of Abernethy malformation complicated by the development of hepatocellular carcinoma. Additionally, we comprehensively reviewed all previously reported cases and highlighted common features that may help in early diagnosis and appropriate management.Patients with Abernethy malformation may have an increased propensity to develop hepatocellular carcinoma. All 5 previously reported cases, plus the three of our patients, have a type 1（complete） shunt suggesting a role for absent portal blood flow in the pathogenesis of hepatocellular carcinoma. Congenital extrahepatic portosystemic shunt should be sought for in cases with raised serum ammonia, hepatic encephalopathy or hepatocellular carcinoma in the absence of cirrhosis.展开更多
Vein of Galen malformations(VGMs)are rare,consisting approximately 1%of intracranial vascular mal-formations,but nearly one third of pediatric vascular mal-formations,which can cause severe morbidity and mortality in ...Vein of Galen malformations(VGMs)are rare,consisting approximately 1%of intracranial vascular mal-formations,but nearly one third of pediatric vascular mal-formations,which can cause severe morbidity and mortality in infants and older children,but more particu-larly in neonates.We presented 2 patients with VGMs which were confirmed by characteristic clinical symptoms and neuroradiological findings(including CT,MRI and/or angiography).One patient underwent embolization fol-lowed by CT,MRI and conventional angiography.The patient’s neurological and cognitive status was normal at present.The other neonate died of congestive heart failure 36 h after birth.展开更多
文摘Congenital extrahepatic portosystemic shunt,also known as Abernethy malformation, is a rare congenital malformation. It causes shunting of blood through a communication between the portal and systemic veins such as a patent ductus venous. We report 3 cases of Abernethy malformation complicated by the development of hepatocellular carcinoma. Additionally, we comprehensively reviewed all previously reported cases and highlighted common features that may help in early diagnosis and appropriate management.Patients with Abernethy malformation may have an increased propensity to develop hepatocellular carcinoma. All 5 previously reported cases, plus the three of our patients, have a type 1（complete） shunt suggesting a role for absent portal blood flow in the pathogenesis of hepatocellular carcinoma. Congenital extrahepatic portosystemic shunt should be sought for in cases with raised serum ammonia, hepatic encephalopathy or hepatocellular carcinoma in the absence of cirrhosis.
文摘Vein of Galen malformations(VGMs)are rare,consisting approximately 1%of intracranial vascular mal-formations,but nearly one third of pediatric vascular mal-formations,which can cause severe morbidity and mortality in infants and older children,but more particu-larly in neonates.We presented 2 patients with VGMs which were confirmed by characteristic clinical symptoms and neuroradiological findings(including CT,MRI and/or angiography).One patient underwent embolization fol-lowed by CT,MRI and conventional angiography.The patient’s neurological and cognitive status was normal at present.The other neonate died of congestive heart failure 36 h after birth.
